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Keywords: MUC15; amino-acid sequence; bovine and human cDNA; splice variant; N-glycosylation.. In addition, the cDNA encoding the human homologue has been isolated and sequenced, thereby

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Isolation and characterization of MUC15, a novel

cell membrane-associated mucin

Lone T Pallesen, Lars Berglund, Lone K Rasmussen, Torben E Petersen and Jan T Rasmussen

Protein Chemistry Laboratory, Department of Molecular and Structural Biology, University of Aarhus, Denmark

The present work reports isolation and characterization of a

highly glycosylated protein from bovine milk fat globule

membranes, known as PAS III Partial amino-acid

sequen-cing of the purified protein allowed construction of

degen-erate oligonucleotide primers, enabling isolation of a

full-length cDNA encoding a protein of 330 amino-acid

residues N-terminal amino-acid sequencing of derived

peptides and the purified protein confirmed 76% of the

sequence and demonstrated presence of a cleavable signal

peptide of 23 residues, leaving a mature protein of 307 amino

acids Database searches showed no homology to any other

proteins A survey of the human genome indicated the

presence of a corresponding gene on chromosome band

11p14.3 Isolation and sequencing of the complete cDNA

sequence of the human homologue proved the existence of

the gene product (334 amino-acid residues) This novel

mucin-like protein was named MUC15 by appointment of

the HUGO Gene Nomenclature Committee The deduced

amino-acid sequences of human and bovine MUC15 dem-onstrated structural hallmarks characteristic for other membrane-bound mucins, such as a serine, threonine, and proline-rich extracellular region with several potential glycosylation sites, a putative transmembrane domain, and a short cytoplasmic C-terminal We have shown the presence

of O-glycosylations, identified N-glycosylations at 11 of 15 potential sites in bovine MUC15, and a splice variant encoding a short secreted mucin Finally, analysis of human and bovine cDNA panels and libraries showed MUC15 gene expression in adult human spleen, thymus, prostate, testis, ovary, small intestine, colon, peripheral blood leukocyte, bone marrow, lymph node, tonsil, breast, fetal liver, bovine lymph nodes and lungs of both species

Keywords: MUC15; amino-acid sequence; bovine and human cDNA; splice variant; N-glycosylation

Mucins are a heterogeneous family of high molecular mass

proteins that are broadly defined by their high content of

carbohydrates (50–90%), which are mainly O-linked but in

some cases also N-linked These glycoproteins are major

constituents of the mucus covering the surfaces of epithelial

organs and they provide selective physical barriers

protect-ing the underlyprotect-ing epithelium Mucins are known to be

expressed in various epithelia Nevertheless, the overall

expression patterns have not been completely elucidated

(reviewed in [1,2]) To date 15 human mucin genes encoding

epithelial mucin type proteins have been identified: MUC1,

-2, -3A, -3B, -4, -5AC, -5B, -6, -7, -8, -9, -11, -12, -13, and -16

[3–15] In addition, two mouse mucin genes, MUC10 and

MUC14, have been isolated ([16], GenBank accession

number NM_016885) Mucins can be divided into at least

two structurally and functionally distinct classes, the

secreted (gel-forming or nongel-forming) mucins and the

membrane-associated mucins

Four of the secreted mucins are encoded by a cluster of genes (MUC2, MUC5AC, MUC5B and MUC6) contained within a 400-kb genomic DNA fragment on chromo-some 11 band p15.5 [17] The MUC7, MUC8 and MUC9 are relatively small mucins expressed in the salivary gland, respiratory tissue and fallopian tube, respectively The family of epithelial membrane-associated mucins includes MUC1, -3, -4, -12, -13 and -16 MUC3, MUC11, and MUC12 have been located to chromosome 7q22 suggesting the presence of yet another cluster of mucin genes It should, however, be noted that only partial sequences are known for the MUC11 and MUC12 genes and that it is possible that they are produced as a result of alternative splicing of a single, large mucin gene [13] Human MUC1 was the first mucin to be cloned and is to date probably the best characterized of the mucins Generally, MUC1 is expressed

on the apical cell surface of nearly all polarized epithelial tissues that line ducts and glands, e.g mammary gland [18] MUC1 is found to be a major constituent of human and bovine milk fat globule membranes (MFGM) surrounding the lipid droplets secreted from the mammary gland epithelial cells [19,20]

Bovine MFGM has been shown to contain another heavily glycosylated mucin-like glycoprotein with high molecular mass, named PAS III Glycoprotein C, Glyco-protein 4, Component II and PAS3, are alternative names that have been used for this glycoprotein as well [21] This poorly characterized glycoprotein has been named accord-ing to its mobility upon separation by SDS/PAGE and ability to stain with periodic acid-Schiff’s reagent (PAS) [22] The protein appears heterodisperse with apparent molecular

Correspondence to J Trige Rasmussen, Protein Chemistry

Laboratory, Gustav Wieds Vej 10C, 8000 Aarhus C, Denmark.

Fax: + 45 86136597, Tel.: + 45 89425093,

E-mail: trige@imsb.au.dk

Abbreviations: PAS, periodic acid-Schiff’s; MFGM, milk fat globule

membrane; MTC, multiple tissue cDNA.

Note: reported nucleotide sequences are available from the EMBL

Nucleotide Sequence Database under the accession numbers

AJ417816, AJ417817 and AJ417818.

(Received 5 December 2001, revised 13 March 2002,

accepted 22 April 2002)

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mass ranging from 95 to over 100 kDa in polyacrylamide

gels Antibody staining of sections from bovine prelactating

and lactating mammary gland using monoclonal and

polyclonal antibodies has shown that PAS III is largely

concentrated on apical surfaces of the mammary epithelial

cells [23]

The present work was initiated in order to isolate and

characterize the bovine mucin-like glycoprotein PAS III A

purification method has been established, together with a

determination of the complete amino-acid sequence

enco-ded by the corresponding cDNA In addition, the cDNA

encoding the human homologue has been isolated and

sequenced, thereby identifying a novel human

transmem-brane mucin gene named MUC15 by appointment of the

HUGO Gene Nomenclature Committee Presence of

O-glycosylation and sites of N-glycosylation in bovine

MUC15/PAS III have been determined, and a splice variant

encoding a short secreted mucin was identified Finally,

PCR on cDNA panels revealed MUC15 expression in a

variety of human tissues

M A T E R I A L S A N D M E T H O D S

Purification of bovine MUC15

MFGM was prepared as described by Hvarregaard et al

[24] using the cream fraction of freshly collected

unpasteur-ized bovine milk samples Bovine MUC15 was purified

from MFGM using a method essentially as the one used for

isolation of bovine MUC1 [20] Briefly, MFGM proteins

were extracted from the membranes using the nonionic

detergent Triton X-100 Extracted proteins were subjected

to cation- and anion-exchange chromatography on

CM-Sepharose and DEAE-CM-Sepharose columns, respectively

(Amersham Pharmacia Biotech, Uppsala, Sweden)

MUC15 containing fractions were dialyzed, freeze-dried,

and finally subjected to further purification by reverse-phase

chromatography using a 1-mL Resource RPC column

(Amersham Pharmacia Biotech) with a gradient of

2-propanol in 20% formic acid MUC15 containing

sam-ples appearing at 48% 2-propanol were collected and

freeze-dried Standard procedures were employed analysing

pro-tein samples by SDS/PAGE using 18% polyacrylamide

gels, and for the staining of proteins using Coomassie

Brilliant Blue R-250 and PAS reagent

Peptide mapping of bovine MUC15

Bovine MUC15 peptides were generated by enzymatic

cleavage of the purified protein with trypsin (Worthington

Biochemical Corp., Lakewood, NJ, USA) for 4 h at 37C

Resulting peptide mixtures were separated by RP-HPLC on

a Vydac C18 column (4· 250 mm, Vydac, Hesperia, CA)

using a linear gradient of acetonitrile (0–80%) in 0.1%

trifluoroacetic acid Selected peptide fractions were further

purified by reverse-phase chromatography on a Sephacil C8

SC 2.1/10 column (Amersham Pharmacia Biotech) using

the same gradient Additional peptides were produced

treating unmodified or deglycosylated MUC15 with five

different proteases independently [Staphylococcus aureus V8

protease, (Worthington Biochemical Corp.), endopeptidase

LysC (Roche, Basel, Switzerland), thermolysin,

chymotryp-sin or elastase (Sigma, St Louis, MO, USA)] and

successively purifying generated peptides by RP-HPLC as described above Deglycosylation of bovine MUC15 was achieved by an initial treatment with neuraminidase (Roche)

in 50 mM ammonium acetate, pH 5.0 at 37C for 18 h After that, N-linked oligosaccharides were removed with peptide-N4-(acetyl-b-glucosaminyl)-asparagine amidase (PNGase F; Roche) in 50 mMsodium phosphate, pH 7.5, 0.5% SDS, 5 mM dithioerythritol, 2% octyl-glycopyrano-side for 18 h at 37C Finally, a part of this material was treated with endo-a-N-acetylgalactosaminidase (O-glycosi-dase; Sigma) in 50 mMsodium citrate, pH 6.0 at 37C for

20 h Purified MUC15 and resolved peptide fragments were subjected to N-terminal amino-acid sequencing by automa-ted Edman degradation by means of an ABI 477 A/120 A Protein Sequencer (Applied Biosystems, Foster City, CA, USA) with online identification of the phenylthiohydantoin derivatives N-glycosylation sites were assigned to aspara-gine residues lacking an identifiable phenylthiohydantoin derivative during amino-acid sequencing of glycosylated samples or showing up as aspartic acid in PNGase F treated MUC15 peptides

Cloning of the bovine MUC15 cDNA by PCR with degenerate primers

Isolation of total RNA from the mammary gland of a lactating Danish Holstein cow was performed by means of

an RNeasy kit (Qiagen, Hilden, Germany) Synthesis of cDNA was performed by oligo(dT) primed reverse tran-scription of the isolated total RNA using M-MLV Reverse Transcriptase (Life Technologies, Inc., Gaithersburg, MD, USA) in accordance with the manufacturer’s instructions Six degenerate oligonucleotides were synthesized corres-ponding to partial bovine MUC15 amino-acid sequences obtained by peptide mapping and N-terminal sequencing of the mature protein (DNA Technology, Aarhus, Denmark): P1, 5¢-GARGARGGICARAARAC-3¢ (forward), corres-ponding to the amino-acid sequence E(24)EGQKT(29) (residues underlined in Fig 1B); P2, 5¢-AARACNATGGA RAAYCA-3¢ (forward), K(40)TMENQ(45); P3, 5¢-TCYT TRTCISWIGTIARRTT-3¢ (reverse), N(54)LTSDKE(60); P4, 5¢-GGYTCRTTICKRTCRTCRTA-3¢(reverse), Y(271) DDRNEP(277); P5, 5¢-CATRTCRTAIGGYTCIGGNG C-3¢ (reverse), A(284)PEPYDM(290); P6, 5¢-GCNGTIGG RTTRTARTA-3¢ (reverse), Y(297)YNPTA(302); where

R¼ A or G, Y ¼ C or T, K ¼ G or T, S ¼ C or

G, W¼ A or T, N ¼ A, G, C or T, and I ¼ deoxyin-deoxyinosine The degenerate primers were employed in PCR amplifications of cDNA performed in a total volume

of 25 lL containing 0.4 mM dNTPs, 2.5 lL 10· PCR buffer, 2.5 U of HotStarTaq polymerase (HotStarTaq Master Mix Kit, Qiagen), 5 lL first-strand cDNA and

4 lM each of the forward and reverse degenerate primers After a 15-min, 95C activation step of the HotStarTaq DNA polymerase, amplification was performed as follows: five cycles of denaturation at 94C for 45 s, annealing at

46C for 45 s and extension at 72 C for 120 s, followed by

35 amplification cycles with an annealing temperature of

50C Obtained PCR products were cloned into pCR 2.1-TOPO cloning vectors using the 2.1-TOPO TA Cloning Kit (Invitrogen, Groningen, the Netherlands) Sequencing inserts from 50 positive clones a single was found to contain

a MUC15 fragment of 62 nucleotides generated with the P2

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and P3 primers From the obtained MUC15 nucleotide

sequence a single specific oligonucleotide primer was

designed: P7, 5¢-CAATCTGTCCCTTTAGA-3¢ (forward)

The major part of the coding sequence was then cloned and

sequenced using PCR as described above with 0.4 lMand

4 lM of the specific P7 primer and degenerate primers

(P4-P6), respectively The cDNA sequence of the bovine

MUC15 was extended in both 5¢ and 3¢ directions by PCR

screening of an oligo(dT) primed mammary gland Uni-ZAP

cDNA library, derived from a lactating Holstein cow

(Stratagene, La Jolla, CA, USA), using MUC15-specific

and library vector primers The full-length cDNA was obtained sequencing overlapping clones and PCR products derived by RT-PCR on the isolated RNA from the Danish Holstein cow The bovine MUC15 cDNA was sequenced

on both strands using a BigDye Sequencing kit and an ABI PRISM 310 Genetic Analyser (Applied Biosystems) Identification of the human MUC15 cDNA

The bovine MUC15 nucleotide sequence was employed in a BLASTn search of the human genome database at NCBI, and a match was found on a Ôchromosome 11 working draft sequence segmentÕ (GenBank accession number NT_008952) Identified partial sequences of the putative human homologue were examined and specific PCR primers were designed enclosing the coding sequence of the bovine protein To investigate the presence of MUC15 expression in epithelial cells of the human mammary gland,

we proceeded to isolate the cellular fraction of human milk samples obtained from four lactating women at different stages in the lactation Samples were collected immediately after milking and stored on ice Milk cells were harvested by centrifugation at 3200 g for 20 min at 4C, and the cellular fraction was washed in NaCl/Pibuffer and processed for total RNA purification using a RNeasy Blood kit (Qiagen) Synthesis of cDNA was performed by oligo(dT) primed reverse transcription of the mRNA isolated from milk cells using M-MLV Reverse Transcriptase Using the specific primers in RT-PCR a 1501 base pair cDNA composite of the human gene was obtained RT-PCR products were purified using a Jetquick PCR Purification Spin Kit (Genomed, Bad Oeynhausen, Germany) and sequenced as described for the bovine counterpart

Detection of an alternatively spliced MUC15 variant First-strand cDNA was prepared from the mammary gland RNA of a Danish Holstein cow using M-MLV Reverse Transcriptase as described above Specific forward and reverse primers were designed to produce a PCR product of

513 bp containing the transmembrane domain: 5¢-CATCC ATAGCAGATAACAGTC-3¢ (forward) and 5¢-TCCCA AAGCTCATGTCATAAG-3¢ (reverse) corresponding to amino-acid residues S(123)SIADNSL(130) and P(287)YD MSFGN(294), respectively (see below) The PCR products were subjected to DdeI restriction enzyme treatment (Roche) following standard procedures, and a second round

of PCR was performed using the same primers Obtained PCR products were ligated into pCR 2.1-TOPO cloning vectors and sequenced on both strands

MUC15 expression analysis MUC15 mRNA expression was examined in a variety of tissues and cell types by PCR screening The screening analysis was performed using commercial multiple tissue cDNA (MTC) panels of fetal and adult human tissues (human MTC Panel II, Cat # K1421-1 and human Immune System MTC Panel, Cat # K1426-1, Clontech, Palo Alto, CA, USA) The panels contained normalized, first-strand cDNA preparations generated from each of the following human tissues and cell types: spleen, thymus, prostate, testis, ovary, small intestine, colon, peripheral

Fig 1 Purification of bovine MUC15 and obtained tryptic peptidemap.

(A) RP-HPLC chromatography of bovine milk fat globule membrane

proteins eluted from the DEAE column Separation was performed on

a 1-mL Resource RPC column with a linear gradient of 0–80%

2-propanol in 20% formic acid at 40 C (dotted line) Proteins were

monitored at 278 nm (solid line) The peaks containing MUC1 and

MUC15 are indicated (B) RP-HPLC separation of peptides generated

by trypsin digestion of bovine MUC15 Peptides were eluted from a

Vydac C18 column using a linear gradient from 0 to 80% acetonitrile

in 0.1% trifluoroacetic acid (dotted line), and monitored at 226 nm

(solid line) Amino-acid sequences of labelled peaks are shown.

Underlining indicates amino-acid residues used for design of

degen-erate oligonucleotide primers.

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blood leukocyte, bone marrow, fetal liver, lymph node, and

tonsil Further tissue specific studies were performed by

PCR screening of oligo(dT) primed cDNA libraries of

bovine lymph node, bovine lung, human lung (Stratagene),

and human breast tissue (Clontech) Specific bovine and

human MUC15 primer sets were employed in the PCR

screening reactions PCR products were separated by

electrophoresis on 1% agarose gels, visualized with

ethi-dium bromide and finally sequenced

R E S U L T S

Purification of bovine MUC15

Bovine MUC15 copurifies with MUC1 during the initial

steps of the protocol designed for isolation of the latter

mucin from MFGM [20] Complete separation was

achieved by RP-HPLC on a Resource RPC column with

a gradient of 2-propanol in 20% formic acid (Fig 1A) The

bovine MUC15 eluted at approximately 48% 2-propanol,

and the purity of this fraction was confirmed by SDS/

PAGE (Fig 2) N-terminal amino-acid sequencing of the

isolated mature bovine MUC15 was performed and

revealed a segment of 30 residues (EEGQKTXTTESTAED

LKTMENQSVPLESKA), which did not show similarity to

any known sequences as revealed by BLASTPand FASTA3

homology searching of databases accessed through the

NCBI and EBI, respectively

Sequence description of bovine MUC15

To obtain sequence information from peptide mapping,

purified bovine MUC15 was subjected to enzymatic

diges-tion with trypsin Generated tryptic peptides were separated

by RP-HPLC, and subjected to N-terminal amino-acid

sequencing (Fig 1B) To enable deduction of the complete

amino-acid sequence of bovine MUC15 by cDNA cloning,

six degenerate oligonucleotide primers were designed from

the acquired partial amino-acid sequences After RT-PCR

on mammary gland mRNA of a Danish Holstein cow a

single MUC15 fragment was cloned and a specific primer

was constructed By additional use of degenerate and

specific MUC15 primers, a full-length cDNA sequence was

obtained Reported nucleotide sequence data are available from the EMBL Nucleotide Sequence Database under the accession number AJ417816

Analysis of the obtained full-length cDNA sequence (3125 nucleotides in total) showed the presence of an open reading frame encoding a protein of 330 amino-acid residues (Fig 3) Approximately 76% of the cDNA-enco-ded amino-acid sequence was confirmed by N-terminal sequencing of the mature protein and enzymatic generated peptides (Fig 3, underlined residues) The proposed trans-lational start codon (ATG) follows a 5¢ untranslated sequence of 120 nucleotides The translational stop codon (TAA), positioned at residues 1111–1113, is followed by a 3¢ untranslated sequence of 1994 nucleotides, including a polyadenylation signal (AATAAA) (position 3085–3090)

Fig 2 SDS/PAGE analysis of purified bovine MUC15 Analysis was performed on 18% Tris/glycine polyacrylamide gels Positions of molecular mass standards are indicated to the left Gels were stained with periodic acid-Schiff’s reagent (PAS) Lane 1, bovine milk fat globule membrane proteins (MFGM); lane 2, fraction from the Resource RPC column containing purified bovine MUC15; lane 3, neuraminidase and O-glycosidase treated bovine MUC15; lane 4, PNGase F treated bovine MUC15; lane 5, neuraminidase treated bovine MUC15.

Fig 3 Alignment of the deduced amino-acid sequences of bovine and human MUC15 Fully conserved residues are indicated with black boxes Amino-acid sequence obtained by peptide mapping and Edman degradation of the bovine protein is underlined Identified bovine N-glycosylation sites are marked with asterisks and arrows indicate the signal peptide and transmembrane region The alignment was performed using the BIOLOGY WORKBENCH 3.2, San Diego Supercomputer Center, University of California, San Diego EMBL Accession Numbers: bovine (AJ417816), human (AJ417818).

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and a poly(A) tail of 18 nucleotides Two alternative

poly(A) signals [A(1259)TAAA and A(1430)ATTAAA]

giving rise to poly(A) tails were observed by PCR-screening

of the bovine mammary gland cDNA library

The N-terminal amino-acid sequencing of purified bovine

MUC15 revealed Glu24 as the initial residue of the mature

protein, showing that the preceding 23 residues comprise a

cleavable signal peptide Computer analysis of the

transla-ted protein sequence suggestransla-ted presence of a single

mem-brane-spanning domain (residues 234–256, Fig 3), giving

rise to a type 1 integral membrane protein spanning the

plasma membrane once The protein appears to be oriented

with an intracellular C-terminal region of 74 residues

(residues 257–330) and an extracellular N-terminal part

(amino acids 24–233, Fig 4) The N-terminal region of

MUC15, rich in serine, threonine and proline residues,

contains 15 consensus motifs for N-glycosylation and

numerous potential O-glycosylation sites

N- and O-glycosylation of bovine MUC15

The calculated average molecular mass of the mature

MUC15 at 33 317 Da is quite distant from the

approxi-mately 100 kDa extrapolated from the electrophoretic

mobility (Fig 2) The heavy glycosylation, suggested by

the staining behaviour of the protein, might explain at least

a part of this discrepancy The carbohydrate might thereby

constitute up to 67% of the relative molecular mass,

although the massive glycosylation most likely affects the

electrophoretic migration of the protein Removal of sialic

acid by neuraminidase resulted in a slight decrease in the

mobility of bovine MUC15 in SDS/PAGE (Fig 2)

Pres-ence of O-linked glycans was shown by incubating

neura-minidase treated protein with O-glycosidase, which reduced

the relative molecular mass (Fig 2) This indicates the

presence of core-1 O-linked glycans, as O-glycosidase

specifically liberates Galb1–3GalNAc from serine and

threonine residues Upon PNGase F treatment, the appar-ent molecular mass of MUC15 shifted from 100 kDa to approximately 80 kDa (Fig 2), demonstrating ample pres-ence of N-linked glycans Hydrolysis of the Asn-oligosac-charide linkage by PNGase F leads to deamination of asparagine to aspartic acid [25] This facilitates identification

of N-glycosylation sites during amino-acid sequencing, as

an Asp-phenylthiohydantoin derivative is seen instead of the unidentifiable glycosylated asparagine derivative Fol-lowing sequence analysis of the generated peptides, 11 of the

15 possible sites in bovine MUC15 showed to contain N-linked glycosylations (marked with asterisks in Fig 3) Identification and cloning of the human MUC15 cDNA

In order to investigate the existence of a human MUC15 homologue, the bovine MUC15 nucleotide sequence was employed in a search of the human genome database, and a similar sequence was located The milk cell fraction of lactating tissue contains bud-off epithelial cells, enabling performance of an indirect assay for expression of this possible human homologue in mammary epithelium RT-PCR was performed on the RNA isolated from the cellular fractions of milk obtained from four lactating women, and expression of a human MUC15 mRNA transcript was shown in all samples Examination of the obtained cDNA sequence (1501 nucleotides in total, EMBL accession number AJ417818) showed the presence of an open reading frame encoding a protein of 334 amino-acid residues (Fig 3)

Analysis of the coding sequence of human MUC15 suggested that it contains a signal peptide (amino acids 1– 23), an extracellular Ser, Thr, Pro, Leu and Asn rich area (residues 24–237) containing 10 N-glycosylation motifs and numerous possible O-glycosylation sites, a transmembrane domain (residues 238–260), and a short cytoplasmic C-terminal (residues 261–334) Thus, the mature human

Fig 4 Schematic representation of MUC15 (A) Schematic representation of the human MUC15 gene Nucleotide positions (in AJ417818) are indicated by numbers Exons and introns are indicated by E and I, respectively Intron sizes are given in parentheses Shaded boxes represent the coding regions whereas white boxes indicate the noncoding regions (B) Schematic representation showing the organization of the bovine MUC15 protein: The 23 amino-acid signal peptide (SP), the extracellular Ser, Thr, and Pro rich region, the transmembrane domain (TM), and the cytoplasmic C-terminal (CYT) Positions of the domains are indicated with amino-acid numbers Identified N-glycosylation sites are marked with hexagons The protein is oriented with an exoplasmic N-terminal and a cytoplasmic C-terminal tail The 50-amino-acid region skipped in the MUC15/S splice variant is shown.

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MUC15 is proposed to comprise 311 amino acids with a

calculated average mass of 33 875 Da

Alignment of the bovine and human MUC15 sequences

showed 67% similarity (Fig 3) The majority of the

differ-ences exist in the extracellular part where similarity with the

bovine mucin is only 59% The similarity rises to 87% in the

transmembrane domain and cytoplasmic area, suggesting

that these regions may be of functional importance

By comparison of the human MUC15 cDNA sequence

with the working draft sequence version of the human

genome, available from the NCBI, homologous sequences

were located on chromosome 11 (p14.3 region) With two

minor exceptions, the derived and genomic sequences were

identical These differences correspond to nucleotide

vari-ations observed at positions 495 (a–g polymorphism) and

827 (t–c polymorphism), the latter causing an amino-acid

change from Ile to Thr (residue 202 in Fig 3) Comparing

the obtained human MUC15 cDNA and the genomic

sequence revealed the boundaries of five exons and four

introns (Fig 4A) The signal peptide and the major part of

the extracellular part are encoded by a single exon (exon 3),

which is followed by a 150-bp exon encoding the

trans-membrane domain (exon 4) Nucleotides encoding the

cytoplasmic domain span exons 4 and 5, which also contain

the stop codon as well as a 274-bp 3¢ untranslated region

Alternatively splicing and expression pattern of MUC15

Database searches showed that MUC15 is widely expressed,

as numerous human EST clones have been isolated from

fetal liver and spleen, fetal ear, placenta, lung, pancreas and

kidney (e.g accession numbers; H53268, BI491080, BG434403, BG485125, AA386131, BG425830) By PCR screening of human MTC panels using MUC15-specific primers we have also demonstrated human MUC15 mRNA expression in a wide range of tissues; adult human spleen, thymus, prostate, testis, ovary, small intestine, colon, peripheral blood leukocyte, bone marrow, lymph node, tonsil, and fetal liver Furthermore, PCR screening of bovine and human cDNA libraries showed the presence of MUC15 mRNA in human breast, bovine mammary gland, bovine lymph nodes and lungs of both species (Table 1) Of the identified ESTs a single clone, isolated from the human lung (GenBank accession number BG485125), appeared to have been derived from an alternative splicing event In agreement with this, 11 of the 19 PCR screening experi-ments revealed a smaller and weaker band in addition to the expected product (Table 1) Therefore, to investigate the possible existence of an alternatively spliced mRNA variant

of MUC15, RT-PCR experiments were performed on total RNA extracted from the mammary gland of a Holstein cow Using MUC15-specific primers flanking the region containing the potential splice site, a major band of 513 bp was amplified by RT-PCR, along with a second shorter and weaker band To specifically amplify the shorter variant in a second round of PCR, the products were subjected to specific enzymatic cleavage with the DdeI enzyme, which should only cut generated products comprising the trans-membrane region Isolation and sequencing of a clone corresponding to the short variant confirmed the presence

of an alternatively spliced form of bovine MUC15 The isolated variant (EMBL accession number AJ417817)

Table 1 Expression of MUC15 in human and bovine tissues and cell types MUC15 mRNA expression was examined by PCR screening of commercial multiple tissue cDNA panels and oligo(dT) primed cDNA libraries and by RT-PCR on RNA isolated from the mammary gland of a Holstein cow and the cellular fraction of human milk samples ND, not detected; NI, not investigated.

Human

Bovine

a

Primer pair: 5¢-AATACCAAAGAAGCCTACAATG-3¢ and 5¢-GTACGAAGTGGAGGTATGTCATC-3¢ b

Primer pair: 5¢-GCCATTT TAGGTGCTATTCTGG-3¢ and 5¢-TATTTTCTTTATCTGAGTTTA-3¢ c

Primer pair: 5¢-CATCCATAGCAGATAACAGTC-3¢ and 5¢-T CCCAAAGCTCATGTCATAAG-3¢ d

Generated PCR products have been additionally verified by nucleotide sequencing.

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showed deletion of a segment of 150 nucleotides,

corres-ponding to the entire exon 4 of the human homologue

encoding the transmembrane domain This variant was

called MUC15/S in analogy with the secreted short variant

of human MUC1 (GenBank accession number AF348143)

Thus, bovine MUC15/S encodes a potential secreted mucin

of 257 amino acids with a calculated molecular mass of

27 842 Da

D I S C U S S I O N

The present paper describes the purification and

character-ization of a hitherto unknown bovine membrane-associated

mucin-like glycoprotein, MUC15, and cloning of a human

homologue The mature protein contains a single

trans-membrane domain, and is proposed to be oriented with a

small intracellular C-terminal part and an extracellular

N-terminal comprising numerous N- and O-glycosylation

sites (Fig 4) Furthermore, database searches performed to

look for other proteins with significant sequence similarity

turned out fruitless

Several features of the isolated bovine glycoprotein

suggest that it is a mucin-type molecule; a high molecular

mass, a high content of carbohydrate, and third expression

at the apical surface in epithelial cells of the mammary

gland Likewise, the deduced amino-acid sequences of both

bovine and human MUC15 resemble the mucins in having

serine, threonine and proline as the predominant amino

acids, however, their high contents of leucine and

aspara-gine is a characteristic shared only with the MUC8, MUC9,

MUC13, and MUC16 Like the membrane-associated

members of the mucin family, MUC15 appears to be

derived from a precursor sequence including a signal

peptide, a serine/threonine/proline-rich extracellular region,

a hydrophobic transmembrane domain and a cytoplasmic

tail Although most structural elements of the

membrane-associated mucins turned out to be present in MUC15, it is

unique in its short extracellular domain and lack of

repetitive segments with the typical mucin tandem repeats

However, lack of tandem repeats is also seen in the

mucin-like glycoproteins mouse MUC14, endomucin-1, and

endomucin-2 [26] Nevertheless, the extracellular region of

MUC15 and traditional mucin tandem repeat domains

share the same characteristics with long extended sequences

devoid of secondary structure and great potential for

extensive glycosylation

Treatment of bovine MUC15 with O-glycosidase

dem-onstrated presence but not extend of O-glycosylation Until

now no specific motif for O-glycosylation has been

identi-fied, however, proline is preferentially positioned in

prox-imity to the glycosylation site and especially in the)1 and/or

+3 positions [27] According to the NetOGlyc server,

predicting mucin-type O-glycosylations using the algorithm

of Nielsen et al [28], the extracellular region of bovine and

human MUC15 offer 22 and 14 O-glycosylation sites,

respectively The majority of these potential O-glycosylation

sites are positioned in the central part of the extracellular

region, which also contains 10 predicted N-glycosylation

motifs in human MUC15 and 15 in the bovine counterpart

Interestingly, doubly glycosylated Asn-Xaa-Ser/Thr motifs

have been reported, illustrating that N-glycosylations do not

hinder O-glycosylation of the surrounding serine and

threonine residues [29] There is limited information

avail-able regarding the actual presence of N-linked oligosaccha-rides in mucins So far, N-glycans have only been identified

on bovine MUC1 together with human MUC2 and MUC5AC [20,30,31] Moreover, N-glycosylations are likely

to be present on human MUC3, MUC4, MUC7, MUC12, MUC13, and MUC16 [10,13–15,32,33] The present inves-tigation shows that bovine MUC15 is N-glycosylated in 11 out of 15 potential sites

Localization of the human MUC15 to chromosome 11p14.3 on the human genome, showed the structure of the gene (Fig 4A) A cluster of four secreted gel-forming mucin genes (MUC2, MUC5AC, MUC5B, and MUC6) has been localized within a 400-kb genomic DNA fragment on chromosome 11 band p15.5, and appears to have originated from a common ancestral gene [34] Despite the location of the MUC15 gene close to the cluster of mucin genes, it does not show the characteristics of this group of secreted gel-forming mucins and therefore presumably has not evolved from the same ancestral gene

Two variant forms of MUC15 cDNA were found to be expressed by the normal bovine mammary gland The short variant of bovine MUC15 (MUC15/S) arises from an alternative splicing event in which a section of 150 nucleotides was spliced out of the mRNA transcript, leading to the synthesis of a protein lacking a 50 amino-acid residues long region covering the transmembrane domain Hence, MUC15/S may represent a secreted nongel forming mucin-type molecule as it does not contain any cysteine-rich regions characteristic for the gel forming mucins [1] Examination of a corresponding alternatively spliced database EST clone of the human lung showed that the missing region of this clone corresponds to exon 4 (Fig 4A) Likewise, nucleotides absent in the bovine MUC15/S variant correspond precisely to exon 4 of the human homologue, indicating a conserved genomic struc-ture of human and bovine MUC15, and exon skipping as a possible explanation for the origin of the splice variant Interestingly, the appearance of alternative soluble variants

of membrane-associated mucins has previously been repor-ted Experiments have shown that the nascent RNA transcripts of the MUC1, MUC3, and MUC4 genes, are spliced in an alternative manner possibly forming soluble molecules that are secreted rather than retained on the cell surface [18,35,36] Recently, the membrane-associated mucin MUC16 was found to be secreted from ovarian tumours and cell lines by an unknown mechanism, however, obtained results indicated that an alternative spliced variant without the transmembrane region might exist [15] More-over, immunohistochemistry studies have demonstrated the MUC13 protein within goblet cell thecae, indicative of secretion in addition to presence on the cell surface [14] To this point, conclusive data showing that the MUC3, MUC4, MUC13 and MUC16 mucins exist in both membrane-associated and nonmembrane soluble forms are still miss-ing Likewise, at present there is no documentation for the existence of the splice variant of MUC15 at the protein level The significance of the potential coexistence of MUC15 splice variants is unclear However, the MUC1/SEC secre-ted form of MUC1, devoid of the transmembrane and cytoplasmic domain, has been found to constitute a cognate binding protein for MUC1/Y, which lacks the tandem repeat region MUC1/SEC interacts with the extracellular domain of MUC1/Y, resulting in the phosphorylation of the

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cytoplasmic domain of MUC1/Y and a concomitant change

in cell morphology [37] These results suggest a mechanism

whereby alternative splicing regulates the relative levels of

both the receptor and its secreted cognate binding protein,

generated from the one and same gene, and thereby also

control the biological effects elicited by the interaction of

these two isoforms Alternatively, it could be speculated that

the secreted isoform of MUC15 may function as a protective

mucin, perhaps as a coconstituent with gel-forming mucins

in mucus, or it may act at the apical cell surfaces as a ligand

for other cell surface molecules

The physiological role of MUC15 is not known, however,

hints might arise from gene expression profiles PCR

screening of human MTC panels and additional cDNA

libraries demonstrated MUC15 and MUC15/S mRNA

expression in a wide range of tissues (Table 1), but at a level

lower than the housekeeping gene,

glyceraldehyde-3-phos-phate dehydrogenase (results not shown) The expression of

mucins is generally thought to be restricted to epithelial

cells Surprisingly, the present data indicate no restriction of

the MUC15 cDNA expression to epithelial cells In

contrast, expression in hematopoietic cells and tissues with

function in the immune system was seen Thereby, it might

be difficult to discriminate between expression by transiting

leukocytes, penetrating vascular endothelium, and the tissue

specific cells MUC1 expression, which is associated most

consistently with epithelial tissues, has also been reported at

mRNA and protein level in peripheral blood lymphocytes,

lymph node samples, bone marrow and in various

hema-topoietic cell lines [18,38,39] In addition, the

membrane-bound MUC13, like the human MUC15, also appears to be

expressed at low levels in prostate, lung, liver, spleen,

peripheral blood leukocytes, lymph node, bone marrow,

testis, and ovary [14] Apparently, although historically

characterized as epithelia-specific, some

membrane-associ-ated mucins are also expressed in immune and

hematopoi-etic cells

A C K N O W L E D G E M E N T S

We express our thanks to Margit Skriver Rasmussen, Parisa

Mabhout and Marian Dyrberg Andersen for technical assistance,

Arla Innovation Centre, Brabrand, Denmark, for supplying the

bovine milk samples, and Department of Pediatrics, Aarhus

University Hospital, Skejby, Denmark for establishing contact to

the human milk donors This work is part of the FØTEK program

supported by the Danish Government and the Danish Dairy

Research Foundation.

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